Using neurofibromatosis-1 to better understand and treat pediatric low-grade glioma.
Relatively little is known about the seminal genetic events that trigger the development of low-grade gliomas in children. Genetically engineered mouse models of the neurofibromatosis-1-inherited tumor predisposition syndrome have identified key intracellular growth control pathways, defined the contribution of the tumor microenvironment to glioma growth, and helped researchers understand the genetic basis for glioma susceptibility. In addition, genetically engineered mouse low-grade glioma models have recently been used in preclinical therapeutic studies to evaluate the efficacy of particular biologically based therapies and to define outcome measures.
Gutmann
David H
DH
Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA. gutmannd@wustl.edu
D000818:Animals; D001932:Brain Neoplasms / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy; D002471:Cell Transformation, Neoplastic / Q000187:drug effects / Q000235:genetics; D002648:Child; D004195:Disease Models, Animal; D004354:Drug Screening Assays, Antitumor / Q000379:methods / Q000639:trends; D005786:Gene Expression Regulation / Q000187:drug effects / Q000235:genetics; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D005910:Glioma / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics / Q000378:metabolism; D009456:Neurofibromatosis 1 / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D015398:Signal Transduction / Q000187:drug effects / Q000235:genetics